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对一名患有库欣病的孕妇进行去氨加压素刺激试验。

Desmopressin Stimulation Test in a Pregnant Patient with Cushing's Disease.

作者信息

Parksook Wasita Warachit, Porntharukchareon Thachanun, Sunthornyothin Sarat

机构信息

Division of General Internal Medicine, Department of Medicine, Faculty of Medicine, Chulalongkorn University, and King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, Thailand.

Division of Endocrinology and Metabolism, Department of Medicine, and Hormonal and Metabolic Research Unit, Excellence Center in Diabetes, Hormone and Metabolism, Faculty of Medicine, Chulalongkorn University, and King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, Thailand.

出版信息

AACE Clin Case Rep. 2021 Nov 26;8(3):105-108. doi: 10.1016/j.aace.2021.11.005. eCollection 2022 May-Jun.

Abstract

OBJECTIVE

Hypothalamic-pituitary-adrenal axis stimulation during pregnancy complicates the investigation of Cushing's syndrome (CS). Our objective was to present the case of a pregnant patient with CS caused by a pituitary tumor in whom the desmopressin stimulation test helped in the diagnosis and led to appropriate management.

CASE REPORT

A 27-year-old woman with 9-week gestation presented with a 2-month history of proximal myopathy. She had high blood pressure, wide purplish striae, and a 1-year history of hypertension and dysglycemia. The 8 am cortisol level was 32.4 μg/dL (normal, 5-18 μg/dL), late-night salivary cortisol level was 0.7 μg/dL (11 pm, normal, <0.4 μg/dL), 24-hour urinary free cortisol levels were 237.6 μg/d (normal, 21.0-143.0 μg/d), and adrenocorticotropic hormone (ACTH) levels were 44.0 pg/mL (8 am, normal, 0-46.0 pg/mL). Nongadolinium-enhanced pituitary magnetic resonance imaging revealed no obvious lesion. The desmopressin stimulation test showed a 70% increase in ACTH levels from baseline after desmopressin administration. Pituitary magnetic resonance imaging with gadolinium revealed an 8 × 8 × 7-mm pituitary adenoma. Transsphenoidal surgery was performed, which revealed the presence of ACTH-positive tumor cells. After tumor removal, the patient carried on pregnancy uneventfully.

DISCUSSION

During pregnancy, ACTH levels may not be an accurate marker to help in the differential diagnosis of CS. Moreover, nongadolinium pituitary imaging might not detect small pituitary lesions.

CONCLUSION

In the present case, the desmopressin stimulation test suggested the diagnosis of Cushing's disease, which subsequently led to successful treatment. This suggests that the desmopressin test serves as a useful test for diagnosing Cushing's disease in pregnant individuals.

摘要

目的

孕期下丘脑-垂体-肾上腺轴受刺激会使库欣综合征(CS)的调查复杂化。我们的目的是介绍一例因垂体瘤导致库欣综合征的孕妇病例,其中去氨加压素刺激试验有助于诊断并促成了适当的治疗。

病例报告

一名妊娠9周的27岁女性,有2个月近端肌无力病史。她患有高血压、宽大的紫色条纹,并有1年高血压和血糖异常病史。上午8点皮质醇水平为32.4μg/dL(正常范围5 - 18μg/dL),午夜唾液皮质醇水平为0.7μg/dL(晚上11点,正常范围<0.4μg/dL),24小时尿游离皮质醇水平为237.6μg/d(正常范围21.0 - 143.0μg/d),促肾上腺皮质激素(ACTH)水平为44.0pg/mL(上午8点,正常范围0 - 46.0pg/mL)。非钆增强垂体磁共振成像未显示明显病变。去氨加压素刺激试验显示,给予去氨加压素后ACTH水平较基线升高70%。钆增强垂体磁共振成像显示一个8×8×7mm的垂体腺瘤。进行了经蝶窦手术,术中发现存在ACTH阳性肿瘤细胞。肿瘤切除后,患者顺利继续妊娠。

讨论

孕期ACTH水平可能不是有助于CS鉴别诊断的准确标志物。此外,非钆垂体成像可能无法检测到小的垂体病变。

结论

在本病例中,去氨加压素刺激试验提示库欣病的诊断,随后促成了成功治疗。这表明去氨加压素试验对诊断孕期库欣病是一项有用的检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54cb/9123553/a1a56637e4f1/gr1.jpg

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